/Denys-Drash syndrome

Denys-Drash syndrome

Rare Disease

MONDO:0008682

Also Known As

Denys Drash syndromeDenys-Drash syndromeDenys-Drash syndrome, autosomal dominant, somatic mutationDrash syndromeWilms tumor and pseudohermaphroditismWilms tumour and pseudohermaphroditismnephrotic syndrome type 4DDSWilms tumor and pseudo- or true hermaphroditismWilms tumour and pseudo- or true hermaphroditismnephropathy associated with male pseudohermaphroditism and Wilms' tumornephropathy associated with male pseudohermaphroditism and Wilms' tumournephropathy, Wilms tumor, and genital anomaliespseudohermaphroditism, nephron disorder and Wilms' tumorpseudohermaphroditism, nephron disorder and Wilms' tumour

Definition

Denys-Drash syndrome (DDS) is a rare urogenital disorder characterized by the association of diffuse mesangial sclerosis (DMS), male pseudohermaphroditism with a 46,XY karyotype, and nephroblastoma.